Prognostic assessment for chronic myelomonocytic leukemia in the context of the World Health Organization 2016 proposal: a multicenter study of 280 patients.

Knowledge on chronic myelomonocytic leukemia (CMML) patients from Argentina and Brazil is limited. Our series of 280 patients depicted an older age at diagnosis (median 72 years old), 26% of aberrant karyotypes, and a prevalence of myelodysplastic (60%) and CMML-0 subtypes (56%). The median overall survival (OS) was 48.2 months for patients in CMML-0 (Ref.), 24.7 months for those in CMML-1 (HR = 2.0, p = 0.001), and 8.8 months for patients in CMML-2 (HR = 4.6, p < 0.001). In the CMML-0 category, median OS were different between myelodysplastic and myeloproliferative subtypes (63.7 vs 21.2 months, p < 0.001); however, no differences were observed within CMML-1 and CMML-2 subtypes (24.7 vs 23.7 months, p = 0.540, and 9.1 vs 8.2 months, p = 0.160). The prognostic impact of 24 variables and 7 prognostic systems was adjusted to the WHO 2016 after validating their usefulness. Multivariate analysis were performed, and the final model revealed Hb ≥ 8 -< 10g/dL (HR 1.7), Hb < 8g/dL (HR 2.8), poor karyotypes (HR 2.1), WHO 2016-CMML-1 (HR 2.1), and CMML-2 (HR 3.5) as independent adverse clinical parameters in our cohort with a borderline influence of platelets count < 50 × 109/L (HR 1.4). We could validate several scoring systems, the WHO 2016 proposal and its prognostic capability, along with accessible covariates, on predicting the outcome in our series of CMML patients from Latin America.

Mutations in chronic myelomonocytic leukemia and their prognostic relevance.

Chronic myelomonocytic leukemia (CMML) is a hematologic malignancy that overlaps with myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS) and tends to transform into acute myeloid leukemia (AML). Among cases of CMML, > 90% have gene mutations, primarily involving TET2 (~ 60%), ASXL1 (~ 40%), SRSF2 (~ 50%), and the RAS pathways (~ 30%). These gene mutations are associated with both the clinical phenotypes and the prognosis of CMML, special CMML variants and pre-phases of CMML. Cytogenetic abnormalities and the size of genome are also associated with prognosis. Meanwhile, cases with ASXL1, DNMT3A, NRAS, SETBP1, CBL and RUNX1 mutations may have inferior prognoses, but only ASXL1 mutations were confirmed to be independent predictors of the patient outcome and were included in three prognostic models. Novel treatment targets related to the various gene mutations are emerging. Therefore, this review provides new insights to explore the correlations among gene mutations, clinical phenotypes, prognosis, and novel drugs in CMML.

Blastic Transformation in Mexican Population With Chronic Myelomonocytic Leukemia.

BACKGROUND: Chronic myelomonocytic leukemia (CMML) is the most aggressive of chronic leukemias, with short overall survival and a high transformation rate to acute leukemia. We investigated the factors related to blastic transformation in a Mexican population treated at a tertiary referral center. PATIENTS AND METHODS: The records of patients with a diagnosis of CMML from 2000 to 2015 were reviewed. A total of 54 patients were included, with a median age of 71 years and an overall survival of 16 months. The patients with incomplete data were excluded. IBM SPSS Statistics, version 21.0, software was used to perform the statistical analysis. RESULTS: The rate of blastic transformation was 33% (18 patients), and the interval time to progression was 9 months (range, 0-87 months). Comparing the patients who did not undergo blastic transformation to those who did, those with progression to acute leukemia tended to be younger (age, 58 vs. 71 years; P = .001), to have a greater peripheral blood blast count (≥ 2% vs. 0%; P = .003), and were more likely to have immature myeloid precursors circulating in the peripheral blood (94% vs. 64%; P = .02). On multivariate analysis, younger age continued to be a statistically significant factor for progression (hazard ratio, 0.97; 95% confidence interval, 0.929-0.987). CONCLUSION: Mexican patients with CMML that progressed to overt acute leukemia were considerably younger, with a higher tumor burden and short overall survival. In this population, it is important to consider more aggressive treatment at diagnosis, focusing on high-dose chemotherapy and hematopoietic stem cell transplantation within a short period.

Chronic myelomonocytic leukemia mimicking hematologic systemic lupus erythematosus.

The relationship between primary hematologic disease and rheumatologic manifestations is well known, especially acute lymphocytic leukemia, lymphomas, plasma cell dyscrasias and myelodysplastic syndrome (MDS). Currently, more has been described about autoimmune manifestations in chronic myelomonocytic leukemia (CMML). Many different clinical scenarios may lead a patient with MDS/CMML initially to seek a rheumatological unit. Autoimmune features such as polymyalgia rheumatic symptoms, myositis, neutrophilic dermatosis, cutaneous vasculitis and positive antinuclear antibodies (ANA) are some examples of clinical presentation of MDS/CMML. Moreover, peripheral cytopenias are a common initial presentation both for systemic lupus erythematous (SLE) and MDS/CMML. The aim of this study was to describe a case of an elderly woman with thrombocytopenia and positivity of antibodies to anti-extractable nuclear antigens (anti-ENA) as initial manifestation of CMML mimicking SLE, and to present some clues that encourage the clinician to perform a bone marrow study in such a clinical scenario.

Déficit adquirido de factor X en leucemia mielomonocítica crónica: reporte de un caso / Isolated factor X deficiency in chronic myelomonocytic leukemia: Report of one case

Bleeding disorders are commonly associated with hemato-oncologic diseases. We report a 68 years old male with a chronic myelomonocytic leukemia derived from a long lasting mielodysplastic syndrome that did not respond to treatment with Azacitidine. The patient was hospitalized due to tonic clonic seizures. A CAT scan showed a hematoma in the frontal lobe. A new assessment of hemostasis revealed an isolated deficiency of Factor X. We speculate that this deficit could be secondary to consumption due to the chronic Myelomonocytic Leukemia.

[Isolated factor X deficiency in chronic myelomonocytic leukemia: Report of one case]. / Déficit adquirido de factor X en leucemia mielomonocítica crónica: reporte de un caso.

Bleeding disorders are commonly associated with hemato-oncologic diseases. We report a 68 years old male with a chronic myelomonocytic leukemia derived from a long lasting mielodysplastic syndrome that did not respond to treatment with Azacitidine. The patient was hospitalized due to tonic clonic seizures. A CAT scan showed a hematoma in the frontal lobe. A new assessment of hemostasis revealed an isolated deficiency of Factor X. We speculate that this deficit could be secondary to consumption due to the chronic Myelomonocytic Leukemia.

Leucemia mielomonocítica crónica / Chronic myelomonocytic leukemia

La leucemia mielomonocítica crónica es un desorden clonal de las células progenitoras hematopoyéticas clasificado como neoplasia mielodisplásica mieloproliferativa. Se caracteriza por la presencia de monocitosis absoluta y persistente en la sangre periférica (> 1.0 x 10 9/L) con la presencia de displasia celular y mieloproliferación en la médula ósea. Los pacientes presentan síntomas relacionados a las citopenias y al estado de hipercatabolismo y al examen físico se encuentra esplenomegalia. El diagnóstico se establece con la combinación del examen de la lámina de sangre periférica, el aspirado medular y la biopsia de médula ósea, el inmunofenotipaje y el estudio molecular de las anomalías que se presentan. El pronóstico de la enfermedad es malo. El tratamiento de elección es el trasplante alogénico de progenitores hematopoyéticos con régimen de acondicionamiento de toxicidad reducida. En alrededor del 30 por ciento de los pacientes la enfermedad se transforma en una leucemia mieloide aguda(AU)

Chronic myelomonocytic leukemia is a clonal hematopoietic stem cell disorder classified as a myelodysplastic/myeloproliferative neoplasm. It is characterized by absolute monocytosis (>1.0 x 10(9)/ L) in the peripheral blood that persists for at least 3 months, with dysplastic and myeloproliferation in the bone marrow. Patients may show symptoms related to cytopenias and hypercatabolic state with splenomegaly. The diagnosis is established by combination of complete blood count, peripheral blood smear, bone marrow aspirate, bone marrow biopsy, immunophenotypic profile and study of molecular abnormalities. The prognosis is bad. The treatment of election is the hematopoietic allogeneic stem cell transplantation with regimen of reduced toxicity. In around 30 percent of these patients the disease transforms to acute myeloid leukemia(AU)

Leucemia mielomonocítica crónica / Chronic myelomonocytic leukemia

La leucemia mielomonocítica crónica es un desorden clonal de las células progenitoras hematopoyéticas clasificado como neoplasia mielodisplásica mieloproliferativa. Se caracteriza por la presencia de monocitosis absoluta y persistente en la sangre periférica (> 1.0 x 10 9/L) con la presencia de displasia celular y mieloproliferación en la médula ósea. Los pacientes presentan síntomas relacionados a las citopenias y al estado de hipercatabolismo y al examen físico se encuentra esplenomegalia. El diagnóstico se establece con la combinación del examen de la lámina de sangre periférica, el aspirado medular y la biopsia de médula ósea, el inmunofenotipaje y el estudio molecular de las anomalías que se presentan. El pronóstico de la enfermedad es malo. El tratamiento de elección es el trasplante alogénico de progenitores hematopoyéticos con régimen de acondicionamiento de toxicidad reducida. En alrededor del 30 por ciento de los pacientes la enfermedad se transforma en una leucemia mieloide aguda...

Chronic myelomonocytic leukemia is a clonal hematopoietic stem cell disorder classified as a myelodysplastic/myeloproliferative neoplasm. It is characterized by absolute monocytosis (>1.0 x 10(9)/ L) in the peripheral blood that persists for at least 3 months, with dysplastic and myeloproliferation in the bone marrow. Patients may show symptoms related to cytopenias and hypercatabolic state with splenomegaly. The diagnosis is established by combination of complete blood count, peripheral blood smear, bone marrow aspirate, bone marrow biopsy, immunophenotypic profile and study of molecular abnormalities. The prognosis is bad. The treatment of election is the hematopoietic allogeneic stem cell transplantation with regimen of reduced toxicity. In around 30 percent of these patients the disease transforms to acute myeloid leukemia...

Multitarget effects of quercetin in leukemia.

This study proposes to investigate quercetin antitumor efficacy in vitro and in vivo, using the P39 cell line as a model. The experimental design comprised leukemic cells or xenografts of P39 cells, treated in vitro or in vivo, respectively, with quercetin; apoptosis, cell-cycle and autophagy activation were then evaluated. Quercetin caused pronounced apoptosis in P39 leukemia cells, followed by Bcl-2, Bcl-xL, Mcl-1 downregulation, Bax upregulation, and mitochondrial translocation, triggering cytochrome c release and caspases activation. Quercetin also induced the expression of FasL protein. Furthermore, our results demonstrated an antioxidant activity of quercetin. Quercetin treatment resulted in an increased cell arrest in G1 phase of the cell cycle, with pronounced decrease in CDK2, CDK6, cyclin D, cyclin E, and cyclin A proteins, decreased Rb phosphorylation and increased p21 and p27 expression. Quercetin induced autophagosome formation in the P39 cell line. Autophagy inhibition induced by quercetin with chloroquine triggered apoptosis but did not alter quercetin modulation in the G1 phase. P39 cell treatment with a combination of quercetin and selective inhibitors of ERK1/2 and/or JNK (PD184352 or SP600125, respectively), significantly decreased cells in G1 phase, this treatment, however, did not change the apoptotic cell number. Furthermore, in vivo administration of quercetin significantly reduced tumor volume in P39 xenografts and confirmed in vitro results regarding apoptosis, autophagy, and cell-cycle arrest. The antitumor activity of quercetin both in vitro and in vivo revealed in this study, point to quercetin as an attractive antitumor agent for hematologic malignancies.

Laboratory diagnosis of chronic myelomonocytic leukemia and progression to acute leukemia in association with chronic lymphocytic leukemia: morphological features and immunophenotypic profile

Chronic myelomonocytic leukemia is a clonal stem cell disorder that is characterized mainly by absolute peripheral monocytosis. This disease can present myeloproliferative and myelodysplastic characteristics. According to the classification established by the World Health Organization, chronic myelomonocytic leukemia is inserted in a group of myeloproliferative/myelodysplastic disorders; its diagnosis requires the presence of persistent monocytosis and dysplasia involving one or more myeloid cell lineages. Furthermore, there should be an absence of the Philadelphia chromosome and the BCR/ABL fusion gene and less than 20% blasts in the blood or bone marrow. Phenotypically, the cells in chronic myelomonocytic leukemia can present myelomonocytic antigens, such as CD33 and CD13, overexpressions of CD56 and CD2 and variable expressions of HLA-DR, CD36, CD14, CD15, CD68 and CD64. The increase in the CD34 expression may be associated with a transformation into acute leukemia. Cytogenetic alterations are frequent in chronic myelomonocytic leukemia, and molecular mutations such as NRAS have been identified. The present article reports on a case of chronic myelomonocytic leukemia, diagnosed by morphologic and phenotypical findings that, despite having been suggestive of acute monocytic leukemia, were differentiated through a detailed analysis of cell morphology. Furthermore, typical cells of chronic lymphocytic leukemia were found, making this a rare finding.

Avaliação do método da imunofenotipagem por citometria de fluxo no diagnóstico das doenças displásicas (SMD, LMA-relacionada à mielodisplasia, LMMC e LMMJ) em adultos e crianças / Evaluation of the method of immunophenotyping by flow cytometry in the diagnosis of dysplastic disease (MDS, AML-related to myelodysplasia,CMML and JMML) in adults and children

As síndromes mielodisplásicas (SMD) se caracterizam por terem uma hematopoese displásica, citopenias e pelo risco de progressão para leucemia mielóide aguda. O diagnóstico baseia-se na clínica e nos achados citomorfológicos da medula óssea (MO) e citogenéticos. Na fase inicial ou quando a MO é hipocelular o diagnóstico é difícil e a citogenética frequentemente é normal. A imunofenotipagem (IMF) tem sido cada vez mais utilizada nos casos de SMD em adultos e pouco explorada na SMD pediátrica. Os nossos objetivos foram: estudar os casos de SMD e doenças correlatas (LMA relacioanda à SMD: LMA-rMD; leucemia mielomonocítica crônica: LMMC e leucemia mielomonocítica juvenil: LMMJ) em adultos e crianças, associando os dados clínicos e laboratoriais aos obtidos pela IMF, que utilizou um painel de anticorpos monoclonais para as várias linhagens medulares. No período compreendido entre 2000 e 2010 foram estudados 87 pacientes (64 adultos e 23 crianças) oriundos do HUPE/UERJ e IPPMG/UFRJ e 46 controles (23 adultos e 20 crianças). Todos os doentes realizaram mielograma, biópsia óssea, citogenética, citoquímica e estudo imunofenotípico. Segundo os critérios da OMS 50 adultos foram classificados como SMD, 11 como LMA-rMD e 3 LMMC. Entre as crianças 18 eram SMD, 2 LMA e 3 LMMJ. Os pacientes adultos com SMD foram divididos em alto risco (n=9; AREB-1 e AREB-2) e baixo risco (n=41; CRDU, CRDM, CRDM-SA, SMD-N e SMD-5q). As crianças com SMD em CR (n=16) e AREB (n=2). Anormalidades clonais recorrentes foram encontradas em 22 pacientes adultos e em 7 crianças. Na análise de IMF foi utilizada a metodologia da curva ROC para a determinação dos valores de ponto de corte a fim de identificar os resultados anormais dos anticorpos monoclonais nos pacientes e nos controles, permitindo determinar a sensibilidade e especificidade desses em cada linhagem. A IMF foi adequada para a análise em todos os pacientes e 3 ou mais anormalidades foram encontradas. A associação da IMF...

Myelodysplastic syndrome (MDS) is characterized by having a dysplastic hematopoiesis, cytopenias and risk of progression to acute myeloid leukemia. The diagnosis is based on clinical and cytomorphologic findings in bone marrow (BM) and cytogenetics. In the initial phase of when the BM is hypocellular, diagnosis is difficult and often with normal karyotype. The flow cytometry immunophenotyping (FCI) analysis has been broadly used in adult MDS cases but is rarely in pediatric MDS. The objectives of this work were: to study MDS cases and correlated diseases (AML with myelodysplasia-related changes; chronic myelomonocytic leukemia - CMML and juvenite myelomonocytic leukemia - JMML) and to correlate laboratorial data to FCI using a panel of monoclonal antibodies for the various marrow lineages in both adult and children. In the period between 2000 and 2010, 87 patients were studied (64 adults and 23 children) coming from HUPE/UERJ and IPPMG/UFRJ and 46 controls (26 adults and 20 children). All patients were submitted to myelogram, bone marrow biopsy, cytogenetic, cytochemistry and immunophenotypic study. According to WHO criteria 50 adults were classified MDS, 12 AML and 3 CMML. Among the children there were 18 MDS, 2 AML, and 3 JMML. MDS adult patients were subdivided into high risk (n=9; RAEB-1 and RAEB-2) and low risk (n=41; RCUD, RCMD-RS, MDS-U and MDS-5q). MDS children were classified as RCC (n=16) and RAEB (n=2). Clonal abnormalities were found in 22 (35%) adult patients and 7 (30%) children. In the analysis of FCI methodology ROC curve was used for determination of cut off abnormalities at monoclonal antibodies in patients and controls which allowed to estimate the sensitivity and specificity of each strain. The FCI was suitable for analysis in all patients and 3 or more abnormalities were found. The association of the FCI increased the sensitivity of morphological analysis in the erythroid lineage from 70 to 97% in adults and from 59 to 86% in children...

Decitabine in myelodysplastic syndromes and chronic myelomonocytic leukemia: Argentinian/South Korean multi-institutional clinical experience.

This multicenter, open-label study evaluated the efficacy and safety of decitabine in patients from Argentina and South Korea with myelodysplastic syndromes or chronic myelomonocytic leukemia. Of 106 patients who received decitabine 20 mg/m(2) intravenously over 1 h once daily for 5 days in 4-week cycles, 99 patients were evaluable after receiving at least two cycles. The overall improvement rate was 35% (19% complete response +4% marrow complete response +4% partial response +8% hematologic improvement). Overall survival at 2 years was 71%. Treatment-related adverse events included febrile neutropenia, thrombocytopenia and bleeding, asthenia, fatigue, and eosinophilia. After complete response (CR), three patients received an allogeneic stem cell transplant. Four patients who relapsed after CR responded to decitabine retreatment. Acute myelogenous leukemia developed during follow-up in 21% of patients. Decitabine in a 5-day outpatient administration schedule was effective and well tolerated in typical clinical practice settings in South America and Asia.

Mesotelioma invasivo de un canino: citología, clínica y hallazgos patológicos / Invasive mesothelioma of canine: cytology, clinical and pathology's Findings

Este reporte describe los hallazgos macro y microscópicos de un mesotelioma invasivo epitelial y mesotelioma bifásico (mixto) maligno en un canino macho de 8 años de edad, raza Pit Bull, que llegó a la Clínica Veterinaria de la Escuela de Medicina Veterinaria y Zootecnia de la Facultad de Ciencias Agropecuarias y Recursos Naturales de la Universidad de los Llanos, cuyo motivo de consulta fue aumento del tamaño del abdomen, ascitis, anorexia, pérdida de peso, depresión, dificultad respiratoria y vómito. Según lo reportado por el propietario, el animal había recibido tratamiento para una falla renal que venía presentando meses atrás. Por el pobre pronóstico y orden del propietario, el animal fue eutanasiado y se remitió para necropsia al Laboratorio de Histopatología Veterinaria de la Universidad, siendo realizada la examinación post mortem. A la necropsia, se halló un tumor que comprometió el pericardio con dos neoplasias ulceradas, el mediastino y peritoneo, con numerosas multifocales o, a menudo, confluentes masas neoplásicas blanquecinas, las cuales estaban esparcidas a través del omento y superficies serosas de los órganos de la cavidad abdominal, como en el riñón derecho, páncreas e igualmente dos masas bilobuladas de color rojo de 1 x 1,5 cm en el tejido subcutáneo de la región inguinal, por extensión directa a través del peritoneo, implantación e invasión del tejido subyacente. Por histopatología, se diagnosticó un mesotelioma invasivo epitelial y mesotelioma bifásico maligno, con metástasis a riñón y páncreas, en los que se encontraron células mesoteliales neoplásicas con figuras mitóticas frecuentes, binucleación e invasión de tejidos submesoteliales infiltrados. En hemograma realizado previo a la necropsia, se encontró en el frotis sanguíneo, células blásticas y núcleos desnudos, hallazgos compatibles con una leucemia mielomonocítica.

This report describes the gross, microscopic features of an invasive epithelioid malignant mesothelioma and biphasic (mixed) malignant mesothelioma in an 8 ¡year ¡old male Pit ¡Bull, arrives to the Veterinary Clinic of the School of Veterinary Medicine and Zootecnia of the Faculty of Agricultural Sciences and Natural Resources of the Universidad de los Llanos, whose consultation reason was a increased of the size of abdomen, ascites, anorexia, weight lost, depression, breathing difficulty, vomit, according to the report of the owner, the animal had received treatment for the renal fail which occurred some months ago. The tumor involved the pericardium with two ulceratived neoplastic masses, pleura, mediastinum, and peritoneum with numerous, multiple foci, white neoplastic masses scattered through the peritoneum, and serous surfaces of the organs of the abdominal cavity as the right kidney, pancreas, and equality two bilobed masses with red color of 1 X 1,5cm in the subcutaneous tissue of the inguinal region by direct invaded through the peritoneum, implantation and direct invasion of the subjacent tissue. By histopathology was diagnosed a invasive epithelioid malignant mesothelioma and biphasic (mixed) malignant mesothelioma, with metastasis to kidney and pancreas, the neoplastic cells with frequent mitotic figures and binucleation that infiltrated submesothelial tissues. In the hemogram realized before the necropsy, we found blastic blood cell frotis and naked shared gross with a mielomonocitic leukaemya.

Treatment of pediatric myelodysplastic syndromes and juvenile myelomonocytic leukemia: the Brazilian experience in the past decade.

BACKGROUND: Therapy strategies for myelodysplastic syndromes (MDS) and juvenile myelomonocytic leukemia (JMML) vary considerably. OBJECTIVE: To review the treatment of Brazilian children who were diagnosed with MDS or JMML in the past decade and reported to the Brazilian Cooperative Group on Pediatric Myelodysplastic Syndromes (BCG-MDS-PED). RESULTS: Of 173 children reported to the BCG-MDS-PED from January 1997 to January 2003 with a suspected diagnosis of MDS or JMML, 91 had the diagnosis confirmed after central review of the bone marrow aspirate and biopsy. Information on previous treatments was available for 78 MDS/JMML patients. Treatment varied from different schedules of low-dose (14%) and standard-dose chemotherapy (50%), granulocyte-colony-stimulating factor (G-CSF 7%), interferon (5%), steroids (2%) and erythropoietin (2%) to allogeneic stem-cell transplantation (SCT) (14%). No survival advantage could be demonstrated based on Hasle's classification or based on treatment. CONCLUSION: This report reflects the current practice in treating Brazilian children with MDS/JMML without specific Cooperative Group guidelines. Treatment modalities were very heterogeneous. The strategies for implementing a national protocol should consider international guidelines and focus on local experience and available resources.

B lineage acute lymphoblastic leukemia transformation in a child with juvenile myelomonocytic leukemia, type 1 neurofibromatosis and monosomy of chromosome 7. Possible implications in the leukemogenesis.

This report describes the case of an 8-month-old infant with a diagnosis of juvenile myelomonocytic leukemia (JMML) and type 1 neurofibromatosis that presented progression to B lineage acute lymphoid leukemia (ALL). The same rearrangement of gene T-cell receptor gamma (TCR gamma) was detected upon diagnosis of JMML and ALL, suggesting that both neoplasias may have evolved from the same clone. Our results support the theory that JMML may derive from pluripotential cells and that the occurrence of monosomy of chromosome 7 within a clone of cells having an aberrant neurofibromatosis type 1 (NF1) gene may be the cause of JMML and acute leukemia.

[Diffuse plane xanthoma with arthritis, serositis, erythema nodosum, vasculitis and myelomonocytic leukemia. Description of a case with autopsy]. / Xantoma plano difuso con artritis, serositis, eritema nodoso, vasculitis y leucemia mielomonocitica. Descripcion de un caso con autopsia.

A 57-year-old woman consulted for a severe xanthelasma followed by a severe and complex systemic disease and died ten years later with xanthomas extended to the face and trunk. Her lipid and cholesterol levels were normal but HDL was below normal values. Diabetes as well as amyloidosis, paraproteinemia and Bence Jones protein in urine were not found. Three years after her initial consultation monocytosis appeared in most but not all of her hemograms. Soon after her spleen was palpable and became very large. She had three bone marrow biopsies showing progressive white cell hypercellularity that was considered as possibly reactive. During her long illness she had recurrent conjunctivitis and systemic complications such as arthritis, pleural and pericardial effusions, vasculitis, sudden deafness with Ménière-like vertigo, erythema nodosum and myositis that responded, at least partially, to steroid treatments though she remained corticoid dependent in order to control her more severe symptoms. Those complications were considered to be probably due to vasculitis or immune phenomena. After a long follow up it was clear that her disease was better classified as diffuse plane xanthoma (DPX) related to a myeloproliferative syndrome and vasculitis. Her final hospitalization was due to a severe esophageal moniliasis and she died a few days later. An autopsy was performed and a chronic myelomonocytic leukemia was the final diagnosis. The ultimate cause of death was a perforated acute duodenal ulcer with peritonitis. Possible relations between DPX, immunologic reactions and myelomonocytic leukemia are discussed.

Peliosis esplénica: Asociación con leucemia mielomonocítica crónica, presentándose con ruptura espontánea del bazo / Splenic peliosis: Asociation with chronic myelomonocitic leukemia, presenting with spontaneous splenic rupture

La pielosis es una rara entidad, de teología poco clara: tóxica, infecciosa relacionada a inmunodepresión (enfermedades consuntivas) inflamatoria o vascular, caracterizada por la presencia de espacios quísticos múltiples, con secuestro hemático, que hasta puede llegar a producir trastornos de la hemostasia, induciendo una extrema fragilidad y por ende la ruptuta facial o espontánea del órgano, a veces sin aumentar el volúmen del mismo. Mucho más rara es su asociación con la leucemia mioelomonocítica crónica (LMMC) sindrome mieloproliferativo crónico de diagnóstico controvertido. Habiendo estudiado en detalle de médula ósea, el baso y la citogenética, concluimos que se trata de una verdadera mieloproloiferación. Sugerimos además un estricto control clínico-hematológico y por imágenes para evaluar adecuadamente a aquellas patologías que se acompañan de grandes esplenomegalias en algún momento de su evolución (AU)

Peliosis esplénica: Asociación con leucemia mielomonocítica crónica, presentándose con ruptura espontánea del bazo / Splenic peliosis: Asociation with chronic myelomonocitic leukemia, presenting with spontaneous splenic rupture

La pielosis es una rara entidad, de teología poco clara: tóxica, infecciosa relacionada a inmunodepresión (enfermedades consuntivas) inflamatoria o vascular, caracterizada por la presencia de espacios quísticos múltiples, con secuestro hemático, que hasta puede llegar a producir trastornos de la hemostasia, induciendo una extrema fragilidad y por ende la ruptuta facial o espontánea del órgano, a veces sin aumentar el volúmen del mismo. Mucho más rara es su asociación con la leucemia mioelomonocítica crónica (LMMC) sindrome mieloproliferativo crónico de diagnóstico controvertido. Habiendo estudiado en detalle de médula ósea, el baso y la citogenética, concluimos que se trata de una verdadera mieloproloiferación. Sugerimos además un estricto control clínico-hematológico y por imágenes para evaluar adecuadamente a aquellas patologías que se acompañan de grandes esplenomegalias en algún momento de su evolución